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中华妇幼临床医学杂志(电子版)

中华妇幼临床医学杂志(电子版) ›› 2021, Vol. 17 ›› Issue (03) : 327 -332. doi: 10.3877/cma.j.issn.1673-5250.2021.03.014

论著

重症先天性膈疝患儿的临床治疗策略
孙柏平1,1, 赵小琳2,2, 武鹏1,1, 杜耿1,1, 徐泉1,1, 罗若谷1,†,1()   
  • 收稿日期:2020-05-03 修回日期:2021-05-08 出版日期:2021-06-01
  • 通信作者: 罗若谷

Clinical treatment strategies of children with severe congenital diaphragmatic hernia

Baiping Sun1,1, Xiaolin Zhao2,2, Peng Wu1,1, Geng Du1,1, Quan Xu1,1, Ruogu Luo1,1,†()   

  • Received:2020-05-03 Revised:2021-05-08 Published:2021-06-01
  • Corresponding author: Ruogu Luo
  • Supported by:
    Project of Health and Family Planning Commission of Shaanxi Province(2016B007)
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引用本文：

孙柏平, 赵小琳, 武鹏, 杜耿, 徐泉, 罗若谷. 重症先天性膈疝患儿的临床治疗策略[J]. 中华妇幼临床医学杂志(电子版), 2021, 17(03): 327-332.

Baiping Sun, Xiaolin Zhao, Peng Wu, Geng Du, Quan Xu, Ruogu Luo. Clinical treatment strategies of children with severe congenital diaphragmatic hernia[J]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2021, 17(03): 327-332.

目的

探讨重症先天性膈疝(CDH)患儿的临床治疗策略。

方法

选择2015年1月至2019年10月，西北妇女儿童医院小儿外科收治的7例重症CDH患儿为研究对象。采用回顾性研究方法收集受试儿一般临床资料、治疗、转归及术后随访结果等进行分析。本研究遵循的程序符合2013年新修订的《世界医学协会赫尔辛基宣言》要求。

结果

本组7例重症CDH患儿临床资料分析结果如下。①一般临床资料：7例患儿中，男、女性患儿分别为4例(No.2、5～7)与3例(No.1、3～4)；1例(No.4)为足月单纯低出生体重儿，1例(No.7)为足月低出生体重合并多系统畸形儿，5例(No.1～3、5～6)为低出生体重早产儿；2例(No.2、7)为采取辅助生殖技术出生婴儿，均合并染色体异常。娩出方式：5例(No.1～4、7)经剖宫产术娩出，2例(No.5～6)自然分娩。4例孕母(No.1～2、6～7)有流产史。②治疗情况：除No.6患儿出生后1、5、10 min Apgar评分均为10分外，其余6例在手术室或产房采取气管插管、高频振荡通气(HFOV)、禁食、胃肠减压治疗。No.1患儿因合并多发复杂畸形，患儿家属放弃手术治疗，3例(No.4～6)经左侧胸后外侧切口入路手术，3例(No.2～3、7)经左上腹切口入路手术进行CDH修补术。③转归：1例(No.1)术前放弃治疗后死亡，3例(No.3～4、7)术后死亡。这4例死亡患儿中，除No.4患儿外，No.1、3、7患儿均合并复杂先天性心脏病及其他系统畸形。3例(No.2、5～6)患儿采取手术治疗后治愈出院，均合并早产、低出生体重、其他非复杂畸形。对这3例痊愈患儿术后随访结果显示，1例(No.2)术后1个月CDH复发，经再次CDH修补术治疗后，膈肌完整，截至随访时未见复发；No.5～6患儿截至随访时，均生长发育良好。

结论

合并复杂先天性心脏病，可能是影响重症CDH患儿预后的主要因素之一。对重症CDH患儿娩出后采取即时气管插管、HFOV、延迟手术修复(DSR)等治疗策略，有助于提高该病患儿手术治愈率。

关键词: 疝，横膈, 插管法，气管内, 高频通气, 先天性膈疝, 高频振荡通气, 延迟手术修复, 儿童
Objective

To explore clinical treatment strategies of children with severe congenital diaphragmatic hernia (CDH).

Methods

A total of 7 cases of children with severe CDH who were treated in Northwest Women′s and Children′s Hospital from January 2015 to October 2019 were selected as research subjects. Their general clinical data, treatment outcomes, prognosis and postoperative follow-up results were collected and analyzed by retrospective method. This study met the requirements of World Medical Association Declaration of Helsinki revised in 2013.

Results

The analysis results of clinical data of 7 children with severe CDH in this study were as follows. ①General clinical data: 4 cases of male children (No.2, 5-7) and 3 cases of female children (No.1, 3-4) respectively. Among them, 1 case (No. 4) was a full-term infant with low birth weight, 1 case (No. 7) was a full-term infant with low birth weight and multisystem malformations, and 5 cases (No.1-3, 5-6) were premature infants with low birth weight. 2 cases (No.2, 7) were infants born with assisted reproductive technology and complicated with chromosomal abnormalities. Delivery mode of pregnant mothers: 5 cases (No. 1-4, 7) were delivered by cesarean section, and 2 cases (No. 5-6) had natural birth. 4 cases of pregnant mothers (No. 1-2, 6-7) had abortion history. ②Treatment effects: except for the No.6 child with 10 scores of Apgar score at 1, 5, and 10 min after birth, the other 6 cases received tracheal intubation and high frequency oscillatory ventilation (HFOV) in the operating room or delivery room, and also received fasting and gastrointestinal decompression therapy. Due to multiple complex malformations, the guardian of No.1 child gave up treatment. The other 6 children were treated with CDH repair surgery, 3 cases (No.4-6) were operated through the left posterolateral thoracic incision, and 3 cases (No. 2-3, 7) were operated through the left upper abdominal incision. ③Prognosis: 4 cases had a poor prognosis during perioperative period, of which 1 case (No. 1) died after giving up treatment before operation, and 3 cases (No. 3-4, 7) died after operation. Among them, except for No.4 child, No.1, 3, and 7 children have complicated congenital heart disease and other system malformations. The other 3 children (No. 2, 5-6) were cured and discharged from hospital after surgery, and they all had premature delivery, low birth weight, and other uncomplicated deformities. Postoperative follow-up results of these 3 cured children showed that 1 case (No. 2) had CDH recurrence in 1 month after operation, and after receiving CDH repair surgery again, the diaphragm was intact and without recurrence of CDH at the end of follow-up. And No. 5-6 children grew well at the end of follow-up.

Conclusions

Complication of congenital heart disease may be one of the main factors affecting the prognosis of children with severe CDH. As to children with severe CDH, treatment strategies such as immediate tracheal intubation, HFOV, and delayed surgical repair (DSR) after delivery may be helpful to improve the surgical cure rate of them.

Key words: Hernia, diaphragmatic, Intubation, intratracheal, High-frequency ventilation, Congenital diaphragmatic hernia, High-frequency oscillatory ventilation, Delayed surgical repair, Child
表1 本组7例重症先天性膈疝患儿临床资料
患儿(No.) 性别 出生体重(g) 出生胎龄(周) 发现CDH时胎龄(周) LHR 合并畸形 出生后1、5 min Apgar评分(分) 通气时间(h) 疝入脏器 转归
术前 术后
1 女 1 620 36+5 20 0.9 复杂先天性心脏病，小脑蚓发育不良 7、9 120 0 —b 死亡
2 男 1 670 36 30 2.4 染色体异常，遗传性呼吸道纤毛运动障碍 7、8 60 74 胃、结肠、小肠、脾 治愈
3 女 1 900 34 25+2 1.7 复杂先天性心脏病，股骨骨折 4、7 12 260 肝左叶、胃、小肠、脾 死亡
4 女 2 340 38+3 33 —a 无 8、9 25 12 小肠、脾、结肠 死亡
5 男 2 450 34+2 32 1.8 肠旋转不良 9、9 72 120 小肠、结肠、脾 治愈
6 男 2 460 36+3 26 3.6 右手拇指畸形，右侧睾丸重度发育不良 10、10 0 120 胃、肠管 治愈
7 男 2 470 39+3 23 1.4 染色体异常，复杂先天性心脏病，多发运动系统畸形 7、8 103 480 胃、小肠、结肠、脾 死亡
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